О. И. Краснопёрова, Елена Николаевна Смирнова, Г. В. Чистоусова
Clinical polymorphism of autoimmune polyglandular syndrome type 1, the presence of atypical forms and a long latent period of the target organ disease determine the difficulties in diagnosing this disease, which leads to late detection of life-threatening conditions and inadequate treatment of such patients. The article presents the results from a retrospective analysis of 7 clinical cases of autoimmune polyglandular syndrome type 1 in children hospitalized between 2006 and 2016. The clinical manifestations of the syndrome included chronic candidiasis (7 patients), hypoparathyroidism (6), chronic adrenal insufficiency (3), and autoimmune hepatitis (3). The atypical clinical signs of the disease (annular erythema, hypoplasia of tooth enamel, sialadenitis) were revealed. All children had high ( 6 norms) antibody titers to interferon , 4 — antibodies to interferon 2. In all cases of chronic adrenal insufficiency and autoimmune hepatitis, the corresponding antibodies were determined in high ( 2 norms) titers. Clinical polymorphism, prolonged monosymptomatic course, prolonged latent period between the debuts of the syndrome manifestations require modern immunological methods of diagnostics and monitoring of the residual function of the target organ for the timely initiation of therapy and prevention of crisis states.
{"title":"Клиническое, иммунологическое и молекулярно-генетическое описание симптомов у детей с аутоиммунным полигландулярным синдромом 1-го типа: описание серии случаев","authors":"О. И. Краснопёрова, Елена Николаевна Смирнова, Г. В. Чистоусова","doi":"10.15690/VSP.V16I1.1694","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1694","url":null,"abstract":"Clinical polymorphism of autoimmune polyglandular syndrome type 1, the presence of atypical forms and a long latent period of the target organ disease determine the difficulties in diagnosing this disease, which leads to late detection of life-threatening conditions and inadequate treatment of such patients. The article presents the results from a retrospective analysis of 7 clinical cases of autoimmune polyglandular syndrome type 1 in children hospitalized between 2006 and 2016. The clinical manifestations of the syndrome included chronic candidiasis (7 patients), hypoparathyroidism (6), chronic adrenal insufficiency (3), and autoimmune hepatitis (3). The atypical clinical signs of the disease (annular erythema, hypoplasia of tooth enamel, sialadenitis) were revealed. All children had high ( 6 norms) antibody titers to interferon , 4 — antibodies to interferon 2. In all cases of chronic adrenal insufficiency and autoimmune hepatitis, the corresponding antibodies were determined in high ( 2 norms) titers. Clinical polymorphism, prolonged monosymptomatic course, prolonged latent period between the debuts of the syndrome manifestations require modern immunological methods of diagnostics and monitoring of the residual function of the target organ for the timely initiation of therapy and prevention of crisis states.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"43 1","pages":"49-53"},"PeriodicalIF":0.0,"publicationDate":"2017-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"73865531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
O. A. Vazemiller, N. S. Ponamareva, D. M. Chubko, I. Y. Ushakov, N. Yu. Makarevskaya, M. M. Sharoglazov, E. V. Basalova, E. P. Kirillova, A. B. Salmina, E. Yu. Emel’yanchik
The article describes the history of a female patient of the first year of life with ischemic heart disease associated with congenital thrombophilia and the development of myocardial infarction. This diagnosis in pediatric practice is casuistry and leads to an adverse outcome more often than in adult patients. The described clinical case draws attention of pediatricians to the alarming symptoms, which allow to suspect thrombophilia in children of the first months of life.
{"title":"Трудный диагноз: острый миокардит или ишемия миокарда у младенца с тромбофилией? Клинический случай","authors":"O. A. Vazemiller, N. S. Ponamareva, D. M. Chubko, I. Y. Ushakov, N. Yu. Makarevskaya, M. M. Sharoglazov, E. V. Basalova, E. P. Kirillova, A. B. Salmina, E. Yu. Emel’yanchik","doi":"10.15690/VSP.V16I1.1695","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1695","url":null,"abstract":"The article describes the history of a female patient of the first year of life with ischemic heart disease associated with congenital thrombophilia and the development of myocardial infarction. This diagnosis in pediatric practice is casuistry and leads to an adverse outcome more often than in adult patients. The described clinical case draws attention of pediatricians to the alarming symptoms, which allow to suspect thrombophilia in children of the first months of life.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"63 1","pages":"54-58"},"PeriodicalIF":0.0,"publicationDate":"2017-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"81270722","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
С. С. Дерябина, Ирина Александровна Тузанкина, Елена Викторовна Власова, М. А. Болков, В. Н. Шершнёв
Mass screening of newborns in Russia for five hereditary diseases does not meet the requirements of the world community for the neonatal screening program. Success in the development of laboratory diagnostic technologies and active introduction of achievements in genetics and molecular biology into medical practice allow for the revision of the list of nosologies included in the national neonatal screening program by replacing the disease or adding new nosologies. The article discusses the possibility of including genetic testing for severe combined immune deficiency in the Newborn Screening Program in Russia. The results from a retrospective study of markers of naive T- and B-lymphocytes (TREC and KREC) in the group of children with immuno-dependent pathology developed in the first year of life are discussed.
{"title":"Неонатальный скрининг на тяжелую комбинированную иммунную недостаточность в России: прекрасное далеко или завтрашняя реальность?","authors":"С. С. Дерябина, Ирина Александровна Тузанкина, Елена Викторовна Власова, М. А. Болков, В. Н. Шершнёв","doi":"10.15690/VSP.V16I1.1696","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1696","url":null,"abstract":"Mass screening of newborns in Russia for five hereditary diseases does not meet the requirements of the world community for the neonatal screening program. Success in the development of laboratory diagnostic technologies and active introduction of achievements in genetics and molecular biology into medical practice allow for the revision of the list of nosologies included in the national neonatal screening program by replacing the disease or adding new nosologies. The article discusses the possibility of including genetic testing for severe combined immune deficiency in the Newborn Screening Program in Russia. The results from a retrospective study of markers of naive T- and B-lymphocytes (TREC and KREC) in the group of children with immuno-dependent pathology developed in the first year of life are discussed.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"15 1","pages":"59-66"},"PeriodicalIF":0.0,"publicationDate":"2017-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85203803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
М. А. Солошенко, Екатерина Иосифовна Алексеева, Т. М. Бзарова, К. Б. Исаева, Рина Валериановна Денисова, О. Л. Ломакина, Е М Кащенко, А. В. Карасёва
This review summarizes the international experience of pneumococcal immunization of patients with juvenile idiopathic arthritis (JIA). The high efficiency and safety of pneumococcal vaccines in children with JIA is shown. Numerous studies have demonstrated an adequate immune response after vaccination even during therapy with immunosuppressive and genetically engineered biological agents drugs. Prevention of the pneumococcal infection helps avoid the development of pneumonia in patients with JIA.
{"title":"Вакцинация пациентов с ювенильным идиопатическим артритом против пневмококка: обзор международного опыта","authors":"М. А. Солошенко, Екатерина Иосифовна Алексеева, Т. М. Бзарова, К. Б. Исаева, Рина Валериановна Денисова, О. Л. Ломакина, Е М Кащенко, А. В. Карасёва","doi":"10.15690/VSP.V16I1.1691","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1691","url":null,"abstract":"This review summarizes the international experience of pneumococcal immunization of patients with juvenile idiopathic arthritis (JIA). The high efficiency and safety of pneumococcal vaccines in children with JIA is shown. Numerous studies have demonstrated an adequate immune response after vaccination even during therapy with immunosuppressive and genetically engineered biological agents drugs. Prevention of the pneumococcal infection helps avoid the development of pneumonia in patients with JIA.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"9 1","pages":"24-28"},"PeriodicalIF":0.0,"publicationDate":"2017-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"87530623","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. M. Запруднов, Array А. Харитонова, Array И. Григорьев
{"title":"К 90-летию образования Союза педиатров России","authors":"A. M. Запруднов, Array А. Харитонова, Array И. Григорьев","doi":"10.15690/VSP.V16I1.1689","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1689","url":null,"abstract":"","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"73 1","pages":"16-17"},"PeriodicalIF":0.0,"publicationDate":"2017-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91296185","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
In the training of modern doctors, great attention should be paid to the formation of communicative competence, since the success of medical practice is not only related to medical knowledge, but also to communicative skills. Doctor is a linguoactive profession: speech and communication skills are necessary for the doctor in medical, preventive, educational, organizational and management activities. Medical rhetoric as a theory and practice of effective speech behavior in a public monologue and dialogue can contribute to the preparation of future pediatricians.
{"title":"Медицинская риторика в формировании коммуникативной компетентности врачей-педиатров","authors":"Гринько Елена Николаевна","doi":"10.15690/VSP.V16I1.1688","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1688","url":null,"abstract":"In the training of modern doctors, great attention should be paid to the formation of communicative competence, since the success of medical practice is not only related to medical knowledge, but also to communicative skills. Doctor is a linguoactive profession: speech and communication skills are necessary for the doctor in medical, preventive, educational, organizational and management activities. Medical rhetoric as a theory and practice of effective speech behavior in a public monologue and dialogue can contribute to the preparation of future pediatricians.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"55 1","pages":"12-15"},"PeriodicalIF":0.0,"publicationDate":"2017-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89316771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Екатерина Иосифовна Алексеева, О. Л. Ломакина, С. И. Валиева, Т. М. Бзарова
The article describes the international experience in monitoring of patients with juvenile idiopathic arthritis. The main goal of most existing international registers of children with juvenile idiopathic arthritis is to evaluate various aspects of the effectiveness and safety of genetic engineering biological drugs in comparison with methotrexate. No results from the analysis of the effectiveness of registers as a tool for long-term monitoring of the disease and medical care for children with systemic juvenile idiopathic arthritis have been found in the available literature.
{"title":"Обзор международных регистров пациентов с системным ювенильным идиопатическим артритом","authors":"Екатерина Иосифовна Алексеева, О. Л. Ломакина, С. И. Валиева, Т. М. Бзарова","doi":"10.15690/vsp.v16i1.1690","DOIUrl":"https://doi.org/10.15690/vsp.v16i1.1690","url":null,"abstract":"The article describes the international experience in monitoring of patients with juvenile idiopathic arthritis. The main goal of most existing international registers of children with juvenile idiopathic arthritis is to evaluate various aspects of the effectiveness and safety of genetic engineering biological drugs in comparison with methotrexate. No results from the analysis of the effectiveness of registers as a tool for long-term monitoring of the disease and medical care for children with systemic juvenile idiopathic arthritis have been found in the available literature.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"68 1","pages":"18-23"},"PeriodicalIF":0.0,"publicationDate":"2017-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82453020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Беляева Ирина Анатольевна, Е. П. Бомбардирова, М. Д. Митиш, Т. В. Потехина, Н. А. Харитонова
The article analyzes the studies of the formation and development of the gut microflora (microbiota) in children and its pathogenetic role. The stages of colonization of the infant’s intestine and the interaction mechanisms between the child’s and mother’s microbiotas are described. The relationship between the formation dynamics of the child's microbiota and the nature of feeding is shown; a sanogenetic role of breastfeeding in relation to the infant’s microflora is noted. The connection of qualitative and quantitative microbiota characteristics with delayed risks of development of metabolic and allergic diseases is described. Particular attention is paid to the analysis of the effect of antibiotics on the formation of microbiota, including in preterm infants. The article describes current approaches to the correction of the gut microbiota disorders with probiotic preparations and substantiates the methods for choosing probiotics. One of these preparations is a probiotic registered in Russia and containing B. lactis BB-12 and S. thermophilus, which is successfully used to correct dysbiosis in newborn infants, including in preterm infants.
{"title":"Онтогенез и дизонтогенез микробиоты кишечника у детей раннего возраста: триггерный механизм нарушений детского здоровья","authors":"Беляева Ирина Анатольевна, Е. П. Бомбардирова, М. Д. Митиш, Т. В. Потехина, Н. А. Харитонова","doi":"10.15690/VSP.V16I1.1692","DOIUrl":"https://doi.org/10.15690/VSP.V16I1.1692","url":null,"abstract":"The article analyzes the studies of the formation and development of the gut microflora (microbiota) in children and its pathogenetic role. The stages of colonization of the infant’s intestine and the interaction mechanisms between the child’s and mother’s microbiotas are described. The relationship between the formation dynamics of the child's microbiota and the nature of feeding is shown; a sanogenetic role of breastfeeding in relation to the infant’s microflora is noted. The connection of qualitative and quantitative microbiota characteristics with delayed risks of development of metabolic and allergic diseases is described. Particular attention is paid to the analysis of the effect of antibiotics on the formation of microbiota, including in preterm infants. The article describes current approaches to the correction of the gut microbiota disorders with probiotic preparations and substantiates the methods for choosing probiotics. One of these preparations is a probiotic registered in Russia and containing B. lactis BB-12 and S. thermophilus, which is successfully used to correct dysbiosis in newborn infants, including in preterm infants.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"120 1","pages":"29-38"},"PeriodicalIF":0.0,"publicationDate":"2017-04-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75488316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Deterioration of development conditions in the perinatal period (starvation of the mother, excess sodium ions in her diet, exposure to glucocorticosteroids, placental insufficiency) programs significant changes in the excretion of sodium ions in the newborn and leads to the development of hypertension. Studies on experimental animals show that unfavourable conditions of intrauterine development can reduce the effective area of glomerular filtration and its rate. These circumstances increase the expression of membrane carriers of sodium ions in the apical membranes, the production of superoxide radicals and enhance the reabsorption of sodium. The article discusses the potential mechanisms of perinatal programming of renal hypertension in humans.
{"title":"Поражение почек и перинатальное программирование артериальной гипертензии: результаты экспериментальных исследований","authors":"О. П. Ковтун, П. Б. Цывьян","doi":"10.15690/VSP.V16I6.1820","DOIUrl":"https://doi.org/10.15690/VSP.V16I6.1820","url":null,"abstract":"Deterioration of development conditions in the perinatal period (starvation of the mother, excess sodium ions in her diet, exposure to glucocorticosteroids, placental insufficiency) programs significant changes in the excretion of sodium ions in the newborn and leads to the development of hypertension. Studies on experimental animals show that unfavourable conditions of intrauterine development can reduce the effective area of glomerular filtration and its rate. These circumstances increase the expression of membrane carriers of sodium ions in the apical membranes, the production of superoxide radicals and enhance the reabsorption of sodium. The article discusses the potential mechanisms of perinatal programming of renal hypertension in humans.","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"32 1","pages":"481-486"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78429316","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"GUIDELINES FOR PREPARATION OF MANUSCRIPTS DESCRIBING CLINICAL CASES IN CHILDREN: SUPPORTING THE CARE AND SCARE PRINCIPLES","authors":"R. Saygitov, A. Ostrovskaya","doi":"10.15690/vsp.v16i5.1798","DOIUrl":"https://doi.org/10.15690/vsp.v16i5.1798","url":null,"abstract":"","PeriodicalId":10919,"journal":{"name":"Current Paediatrics","volume":"100 1","pages":"352-357"},"PeriodicalIF":0.0,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85040594","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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